lipid storage diseases

lipid storage diseases

A group of rare conditions–eg, Fabry's disease, Niemann-Pick disease, and sea-blue histiocytosis syndrome, which are often fatal in early childhood, usually due to a catabolic defect of lipid metabolism and characterized by the accumulation of lipids in one or more organs. See Pseudo-Gaucher's disease, Sphingolipidosis.

The exogenous type is more common and associated with aspiration or inhalation of fatty substances, whereas the endogenous type usually occurs as secondary bronchial obstruction caused by tumors, bacterial infections, bronchiolitis obliterans, and lipid storage diseases [3-5].

Niemann-Pick disease (NPD) refers to a group of inherited metabolic disorders known as the leukodystrophies or lipid storage diseases, in which harmful quantities of a fatty substance (lipids) accumulate in the spleen, liver, lungs, bone marrow, and the brain.

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